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 Pediatric Pulmonology 
  • Post-infectious bronchiolitis obliterans: Can CT scan findings at early age anticipate lung function?
  • The image findings of post-infectious bronchiolitis obliterans (PIBO) have been described, however, we do not know if such findings can predict lung function (LF) deterioration with increasing patient age. Aim: To assess whether computed tomography (CT) abnormalities detected at an early stage of the disease can anticipate abnormal LF a decade later in children with PIBO.We compared CT scans of 21 children with PIBO, done within their first 3 years of life, and their actual LF. To evaluate CT scans we used a modified Bhalla score and, for LF, FEV1 as percentage of predicted values. We calculated prevalence ratios (PRs) by comparing the proportion of patients with worst CT score and worst LF, with the proportion of those with best CT score and worst LF.PR was 1.17 (CI 1.02; 1.34, P = 0.02).The CT finding early in the life of children with PIBO, when assessed by the Bhalla, score seem to anticipate future LF status. Pediatr Pulmonol. © 2010 Wiley-Liss, Inc. more >>

  • Churg-Strauss syndrome in a 14-year-old girl
  • No Abstract. more >>

  • Bronchoscopic instillation of activated recombinant factor VII to treat diffuse alveolar hemorrhage in a child
  • No Abstract. more >>

  • Expression and function of phosphodiesterases in nitrofen-induced congenital diaphragmatic hernia in rats
  • Congenital diaphragmatic hernia (CDH) is an anomaly associated with pulmonary hypoplasia and pulmonary hypertension (PH). The limited efficacy of current approaches to treat PH in CDH, including inhaled nitric oxide (NO), drives the search for other therapies. Phosphodiesterases (PDEs) degrade cyclic nucleotide second messenger cAMP and cGMP downstream of NO thereby limiting the vasodilatory response to NO.To identify therapeutic targets by cataloguing the expression and function of PDE isoforms in the pulmonary vasculature in nitrofen-induced CDH in fetal rats.Quantitative RT-PCR revealed PDE1-5 and PDE9 mRNA expression in pulmonary arteries (PAs) of control and nitrofen-induced CDH term fetal rats. In this order of potency, the PDE inhibitors Sildenafil (PDE5) > EHNA (PDE2) > Rolipram (PDE4) > Cilostamide (PDE3) all dilated isolated third generation PA after pre-constriction with the thromboxane analog U46619. Hyperoxic pre-incubation of PAs significantly attenuated vasodilatation induced by the PDE5 inhibitor Sildenafil (65% vs. 33%, P < 0.004). CDH PAs dilated significantly less to PDE2 inhibitor EHNA compared to control (51% vs. 72%, P < 0.05). Subsequently PDE2 protein expression was higher in PAs of CDH animals.Most PDE isoforms exist in the PAs of fetal rats and their inhibition causes pulmonary vasodilatation. PDE5 inhibition was the most potent vasodilator, however, there were no differences between groups. PDE5-induced vasodilatation was attenuated by hyperoxic pre-incubation. PDE inhibitors might be considered therapeutic targets in combination with iNO in neonates with CDH. Pediatr Pulmonol. © 2010 Wiley-Liss, Inc. more >>

  • Is home spirometry useful in diagnosing asthma in children with nonspecific respiratory symptoms?
  • Variation of lung function is considered to be a hallmark of asthma. Although guidelines recommend measuring it as a diagnostic tool for asthma, the usefulness of this approach has not been studied in children.To assess the usefulness of home spirometry in children with nonspecific lower respiratory tract symptoms, to diagnose or exclude asthma.In school-aged children, referred by their general practitioner because of chronic respiratory symptoms of unknown origin, the diagnosis of asthma was made or excluded by a pediatric pulmonologist (gold standard), based on international guidelines and a standardized protocol. Additionally, children measured peak expiratory flow (PEF) and forced expiratory flow in 1 sec (FEV1) twice daily for 2 weeks on a home spirometer, from which diurnal variation was calculated. These results (index test) were not revealed to the pediatric pulmonologist. The value of home spirometry to diagnose asthma was calculated.Sixty-one children (27 boys) were included (mean age: 10.4 years; range: 6-16 years). Between asthma and no asthma, the mean difference in PEF variation was 4.4% (95% CI: 0.9-7.9; P = 0.016) and in FEV1 variation 4.5% (95% CI: 1.6-7.4; P = 0.003). Sensitivity and specificity, based on the 95th-centile of the reference values for PEF and FEV1 variation (12.3% and 11.8%, respectively) were 50% and 72% for PEF variation and 45% and 92% for FEV1 variation. The likelihood ratio was 1.8 for PEF and 5.6 for FEV1.The contribution of home spirometry in the diagnostic process for asthma in schoolchildren with nonspecific respiratory symptoms is limited. Pediatr Pulmonol. © 2010 Wiley-Liss, Inc. more >>

  • Serum myeloperoxidase in wheezing infants: no association with later asthma
  • No Abstract. more >>

  • Late presentation of congenital diaphragmatic hernia in patients with cystic fibrosis
  • Late presentation of congenital diaphragmatic hernia (CDH) is unusual, especially in patients with cystic fibrosis (CF). To our knowledge, cases of CDH in CF patients and the combined effects on lung function have not been previously described. Here we report two cases of late presenting CDH in CF patients and describe effects on lung function. Late presentation of CDH in CF patients can cause gastrointestinal or respiratory symptoms and requires a high index of suspicion as well as proper interpretation of imaging. In patients with CF and CDH, lung function abnormalities could include obstructive, restrictive defects, or combined defects. Pediatr Pulmonol. ©2010 Wiley-Liss, Inc. more >>

 ScienceDirect Publication: Paediatric Respiratory Reviews 
  • Contraception, Communication and Counseling for Sexuality and Reproductive Health in Adolescents and Young Adults with CF
  • Publication year: 2010
    Source: Paediatric Respiratory Reviews, In Press, Corrected Proof, Available online 6 March 2010
    Anna, Tsang , Carmel, Moriarty , Susan, Towns
    With survival now into the fourth decade and rapid growth of the adolescent and adult population of people with cystic fibrosis CF 1 sexual and reproductive health issues are integral to the management of adolescents and adults with CF. Education and counseling for sexual health related issues must be included in the daily routine of CF care. With advances in genetic counseling, contraception, assisted reproductive technology and collaborative management adolescents and young adults with CF realizing their sexual and reproductive potentials safely and realistically can be possible . more >>

  • Urinary Incontinence in Adolescent Females with Cystic Fibrosis
  • Publication year: 2010
    Source: Paediatric Respiratory Reviews, In Press, Corrected Proof, Available online 4 March 2010
    Gail, Nankivell , Patrina, Caldwell , Jennifer, Follett
    Urinary incontinence has been found to be common in girls and women with cystic fibrosis (CF)[1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11] and [12]. This often can have a marked impact on daily activities and quality of life[1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11] and [12]. It may also lead to potential deterioration of respiratory status due to reduced adherence to forced expiratory manoeuvres (cough, lung function tests and exercise) by the girls trying to avoid occurrence of urinary incontinence (UI)11. Analysis of the literature is limited by the fact that different... more >>

  • Fractals for physicians
  • Publication year: 2010
    Source: Paediatric Respiratory Reviews, In Press, Corrected Proof, Available online 4 March 2010
    Cindy, Thamrin , Georgette, Stern , Urs, Frey
    There is increasing interest in the study of fractals in medicine. In this review, we provide an overview of fractals, of techniques available to describe fractals in physiological data, and we propose some reasons why a physician might benefit from an understanding of fractals and fractal analysis, with an emphasis on paediatric respiratory medicine where possible. Among these reasons are the ubiquity of fractal organisation in nature and in the body, and how changes in this organisation over the lifespan provide insight into development and senescence. Fractal properties have also been shown to be altered in disease and even to... more >>

  • Urinary Incontinence in Adolescent Females with Cystic Fibrosis
  • Publication year: 2010
    Source: Paediatric Respiratory Reviews, In Press, Corrected Proof, Available online 4 March 2010
    Gail, Nankivell , Patrina, Caldwell , Jennifer, Follett
    Urinary incontinence has been found to be common in girls and women with cystic fibrosis (CF)[1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11] and [12]. This often can have a marked impact on daily activities and quality of life[1], [2], [3], [4], [5], [6], [7], [8], [9], [10], [11] and [12]. It may also lead to potential deterioration of respiratory status due to reduced adherence to forced expiratory manoeuvres (cough, lung function tests and exercise) by the girls trying to avoid occurrence of urinary incontinence (UI)11. Analysis of the literature is limited by the fact that different... more >>

  • Fractals for physicians
  • Publication year: 2010
    Source: Paediatric Respiratory Reviews, In Press, Corrected Proof, Available online 4 March 2010
    Cindy, Thamrin , Georgette, Stern , Urs, Frey
    There is increasing interest in the study of fractals in medicine. In this review, we provide an overview of fractals, of techniques available to describe fractals in physiological data, and we propose some reasons why a physician might benefit from an understanding of fractals and fractal analysis, with an emphasis on paediatric respiratory medicine where possible. Among these reasons are the ubiquity of fractal organisation in nature and in the body, and how changes in this organisation over the lifespan provide insight into development and senescence. Fractal properties have also been shown to be altered in disease and even to... more >>

  • Pregnancy and Cystic Fibrosis
  • Publication year: 2010
    Source: Paediatric Respiratory Reviews, In Press, Corrected Proof, Available online 25 February 2010
    Edmund M.T., Lau , Carmel, Moriarty , Robert, Ogle , Peter T., Bye
    The management of a pregnancy in a woman with cystic fibrosis is usually achieved with successful outcomes for mother and child with appropriate mutidisciplinary care. The process begins prior to conception and requires frequent monitoring of the mother's respiratory staus, level of glycaemic control and obstetric welbeing. Recent reports have suggested that pregnancy can be managed without a persisting decrement in lung function beyond what may be expected in women with cystic fibrosis who are not pregnant. With the increasingly positive outcomes for people with cystic fibrosis, it is likely that more couples will chose to pursue pregnancy, cognisant of... more >>

  • Oesophageal atresia and tracheo-oesophageal fistula: current management strategies and complications
  • Publication year: 2010
    Source: Paediatric Respiratory Reviews, In Press, Corrected Proof, Available online 23 February 2010
    Andrew J.A., Holland , Dominic A., Fitzgerald
    The successful operative management of oesophageal atresia and tracheo-oesophageal atresia has been available for approximately 70 years. During this time neonatal intensive care has evolved, surgical techniques have improved and consequently near 100% survival for this condition may now be achieved. In keeping with promising results, the co-morbidities of the condition have gained increasing recognition. In this article, the clinical course from antenatal assessments, neonatal surgery and co-morbidities from infancy to adulthood are reviewed to provide a broad overview of the condition. more >>

  • The Role of Heliox in Paediatric Respiratory Disease
  • Publication year: 2010
    Source: Paediatric Respiratory Reviews, Volume 11, Issue 1, March 2010, Pages 46-53
    Marie D., Frazier , Ira M., Cheifetz
    Helium-oxygen (heliox) gas mixtures have been studied for over 70 years as an adjunctive therapy for airway obstruction in a variety of respiratory diseases. The medical use of heliox is based on the physical properties of helium as its low density makes it advantageous in promoting more efficient flow through narrowed passages. Clinical evidence of the efficacy of heliox in treating paediatric respiratory diseases is increasing in the medical literature. This article consists of a comprehensive review of the literature investigating the utility of heliox in the treatment of paediatric respiratory disorders, including upper and lower airway obstruction, mechanical ventilation,... more >>

  • The Role of Heliox in Paediatric Respiratory Disease
  • Publication year: 2010
    Source: Paediatric Respiratory Reviews, Volume 11, Issue 1, March 2010, Pages 46-53
    Marie D., Frazier , Ira M., Cheifetz
    Helium-oxygen (heliox) gas mixtures have been studied for over 70 years as an adjunctive therapy for airway obstruction in a variety of respiratory diseases. The medical use of heliox is based on the physical properties of helium as its low density makes it advantageous in promoting more efficient flow through narrowed passages. Clinical evidence of the efficacy of heliox in treating paediatric respiratory diseases is increasing in the medical literature. This article consists of a comprehensive review of the literature investigating the utility of heliox in the treatment of paediatric respiratory disorders, including upper and lower airway obstruction, mechanical ventilation,... more >>

  • Editorial
  • Publication year: 2010
    Source: Paediatric Respiratory Reviews, In Press, Corrected Proof, Available online 25 January 2010
    Dominic A., Fitzgerald more >>

  • The management of evolving bronchopulmonary dysplasia
  • Publication year: 2010
    Source: Paediatric Respiratory Reviews, In Press, Corrected Proof, Available online 25 January 2010
    Sven M., Schulzke , J. Jane, Pillow
    Bronchopulmonary dysplasia (BPD) is associated with increased mortality and significant long-term cardiorespiratory and neurodevelopmental sequelae. Treatment of evolving BPD in the neonatal intensive care unit (NICU) is challenging due to the complex interplay of contributing risk factors which include preterm birth per se, supplemental oxygen, positive pressure ventilation, patent ductus arterious, and pre- and postnatal infection. Management of evolving BPD requires a multimodal approach including adequate nutrition, careful fluid management, effective and safe pharmacotherapy, and respiratory support aiming at minimal lung injury. Among pharmacological interventions, caffeine has the best risk-benefit profile. Systemic postnatal corticosteroids should be reserved to ventilated infants... more >>

  • Normal Development of the Lung and Premature Birth
  • Publication year: 2010
    Source: Paediatric Respiratory Reviews, In Press, Corrected Proof, Available online 25 January 2010
    Lucia J., Smith , Karen O., McKay , Peter P., van Asperen , Hiran, Selvadurai , Dominic A., Fitzgerald
    The following review focuses on the normal development of the lung from conception to birth. The defined periods of lung development–Embryonic, Pseudoglandular, Canalicular, Saccular and Alveolar–will be explored in detail in relation to gestational age. Cellular differentiation, formation of the conducting airways and respiratory zone and development of the alveoli will be reviewed. Pulmonary vascular development will also be examined within these periods to relate the formation of the blood-air barrier to the lungs for their essential function of gas exchange after birth. The development of the surfactant and cortisol systems will also be discussed as these need to be... more >>

  • Oxygen Titration Strategies in Chronic Neonatal Lung Disease
  • Publication year: 2010
    Source: Paediatric Respiratory Reviews, In Press, Corrected Proof, Available online 25 January 2010
    Robert, Primhak
    The history of oxygen therapy in neonatology has been littered with error. Controversies remain in a number of areas of oxygen therapy, including targets and strategies in supplemental oxygen therapy in Chronic Neonatal Lung Disease (CNLD). This article reviews some of these controversies, and makes some recommendations based on the available evidence. In graduates of neonatal units who are left with CNLD, oxygen saturation should be kept above 93-95%, with levels below 90% being avoided as far as possible. Titration of oxygen should be done using oximetry recordings which include periods of different activities. Weaning of oxygen supplementation should only... more >>

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